Malignant fibrous histiocytoma occurs most commonly in the extremities and trunk, but rarely in the visceral organs. Malignant fibrous histiocytoma of the small intestine is an extremely rare condition. To our knowledge, only ten cases of primary tumors involving the small intestine have been described up to now. Among them only one case has been reported to lead to intussusception. This case report documents the appearance of a storiform-pleomorphic type primary malignant fibrous histiocytoma of the ileum, which led to intussusception, in a 58-year-old man with a 3-month history of dyspepsia, weight loss, general fatigue, and nonspecific abdominal pain. The patient was well with no sign of disease at 8 months after surgery. The clinical and pathologic characteristics of malignant fibrous histiocytoma of the small intestine as found in the literature are also reviewed.

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